PRETEXT II-III multifocal hepatoblastoma: significance of resection of satellite lesions irrespective of their disappearance after chemotherapy

To determine the outcomes of resection of satellite lesions in PRE-treatment Tumor EXTension (PRETEXT) II and III multifocal hepatoblastoma irrespective of their disappearance after chemotherapy. To compare the overall outcomes of multifocal and unifocal hepatoblastoma. Fourteen patients with PRETEXT II (n = 7) and III (n = 7) multifocal hepatoblastoma treated between April 2006 and July 2014 were analyzed and their outcomes were compared with PRETEXT II and III unifocal hepatoblastoma treated in the similar period. Satellite lesion or the affected segments with disappeared satellite lesions were resected in 11 patients. Amongst them, all relapses were distant except one in the liver. In contrast, two of three patients developed liver relapse when the affected segments were not resected. None of the patients receiving intensive chemotherapy based on SIOPEL-3 guidelines developed a relapse. The 3-year event-free and overall survival were 38.6 and 42.9 % in multifocal hepatoblastoma and 86.4 and 92.4 % in unifocal hepatoblastoma (p = 0.001). Multifocality (p = 0.002) and AFP > 10,000 after induction chemotherapy significantly affected event-free survival (p = 0.01). Multifocal hepatoblastoma is associated with poor outcomes as compared to unifocal hepatoblastoma. These preliminary observations of relapse and the role of chemotherapy intensification deserve further study in a multicenter controlled trial setting.